RESULTS:
Hemangiomas, formed by arteriovenous connections grouped within spaces of flow voids, are the most frequent benign tumors of childhood and within these, at the vascular level, the most common in the head and neck, generating alterations in children’s development, both physiological as psychosocial (4,8). Its prevalence reaches up to 4-10% in the child population, without being able to have data regarding its prevalence in relation to its prenatal diagnosis (4,8) Anatomically, when these lesions are located in Superficial dermis are called superficial hemangiomas, manifesting as raised, red lesions; when they are located in the reticular dermis or in the subcutaneous cellular tissue, they appear as raised masses covered with normal or bluish-colored skin, known as deep hemangiomas. When the same superficial and mixed components coexist in the same lesion, hemangiomas are called mixed hemangiomas (4).
Hemangiomas can also be divided into two genetically distinct subtypes, infantile and congenital, and can also present as an isolated event or in a syndromic association (4). Infantile hemangiomas are red-blue protruding lesions that can appear up to 4 weeks after birth and continue to develop 5 to 6 months after it, in rapid growth that can even lead to doubling in size, all this to later gradually involute from the very beginning from 6 to 12 months of life and up to 4 years (2,4). Its proliferation will also depend on the phase in which the lesion is located, which can be proliferative in which 80% of hemangiomas reach their maximum volume at 5 months (early proliferative phase) or up to 9 months (early proliferative phase). late proliferative), this is followed by the phase of partial regression and total regression (8). This type of hemangiomas can be divided into localized (single, focal) and segmental (plaque-shaped and multifocal) lesions (1,9).
On the other hand, congenital hemangiomas are present at birth and can be rapidly involuting, partially involuting, or non-involuting. The appearance of these lesions occurs in the twelfth week of gestation, which is why its prenatal diagnosis is possible if the ultrasound evaluation is carried out by trained personnel (1). Commonly, the skin manifestations of neonatal hemangiomas are related to liver manifestations, which is why it is always necessary to actively search for similar lesions at the liver in ultrasound assessments (4).
A retrospective study carried out at the University of Chile with 174 patients diagnosed with infantile hemangioma found that 61% of the lesions were located on the head and neck, 21% on the trunk, 10% on the upper limbs, 4% on the lower limbs and 3% in the anogenital region. In these patients, the presence of arteriovenous shunts was also identified in 20% of the lesions and direct afferent branches of the main regional arteries in 15%, which were associated with delayed involution of the lesions in some patients and hindered therapeutic response (8).
The use of ultrasound, especially in the prenatal diagnosis of this type of lesions, seeks to establish the presence of secondary obstructive compromise, especially of the airway that could configure a problem at the time of birth, establishing even the need to perform a cesarean section with the EXIT procedure. (1). Likewise, the evaluation of lesions through the application of Doppler flow helps to classify lesions into low-flow and high-flow lesions, thus predicting their behavior and the response to drug treatment or the appropriate moment for postnatal surgical management (4, 9).
The introduction of fetal echocardiography with its basic cuts and especially the cut of 3 vessels and trachea (3VT) in routine prenatal ultrasound has also made it possible to detect the presence of venous anomalies such as persistent left superior vena cava, this especially when it is related to other congenital heart diseases (CHD), aneuploidies, heterotaxy and extracardiac anomalies (6,10)
PLSVC has a prevalence in the general population of 0.3-0.5%, which increases to 4-8% in patients with congenital heart disease (5,10,11). It has its anatomical origin at the junction of the left jugular and left subclavian veins, running anterior to the aortic arch and the left pulmonary artery to the lateral border of the left atrium to finally drain into the coronary sinus in 90% of cases and in 10% in the left atrium. Dilation of the coronary sinus then becomes one of the main ultrasound signs of PLSVC in the 4-chamber view, as well as the presence of a fourth vessel to the left of the pulmonary trunk and the ductus arteriosus or the appearance of abnormally large vessels ordered (from right to left: aorta, pulmonary artery, PLSVC) in the 3VT section (5,6,12).
A prospective cohort study sought to assess the outcomes of patients with isolated prenatally diagnosed PLSVC compared with those associated with other abnormalities. A total of 256 fetuses were included in this, of which 113 fetuses entered the group with isolated PLSVC; none of these fetuses had adverse neonatal outcomes, however 10 cardiac abnormalities (8.8%) and five extracardiac abnormalities (4.4%) were subsequently diagnosed in the postnatal period. In the group of fetuses with extracardiac anomalies and cardiac anomalies of the 143 analyzed fetuses, 27 of them with septal defects, 23 with conotruncal anomalies, and 17 cases of obstructive disease of the left ventricular outflow tract were detected, setting heart disease as the most common abnormalities. In the study, 11 postnatal deaths were identified, 7 in the neonatal period and 4 after postnatal cardiac surgery; all deaths were attributed to complications from associated pathologies, since the presence of PLSVC could not be related as a cause of perinatal death (7,10)
In relation to aneuploidies, a retrospective study in which 95 cases of patients with PLSVC were analyzed and found a prevalence of 13.5% of aneuploidies in their population. Similarly, it was identified that isolated cases of PLSVC had better obstetric outcomes than non-isolated cases, a group in which the presence of cardiac and extracardiac anomalies worsened neonatal outcomes (11). One of the anomalies that has also been related to PLSVC is the VACTERL anomaly, which has been analyzed in several studies, finding an incidence of association ranging from 3.2% to 25% (13).
Only one of the articles reviewed reported a 57-year-old male who attended the emergency department for chest pain and in whom an irregular mass in the pericardial chamber was documented during echocardiography. During the surgical procedure, it was established that this mass was a cardiac hemangioma measuring 110x65x45 in the wall of the right atrium that seemed to involve part of the interatrial groove; additionally, the lesion presented a communication towards a 3 mm diameter IPVC between the coronary sinus and hemangioma (14).